Arrhythmogenic Right Ventricular Cardiomyopathy in a Child with Dandy-Walker Syndrome: Sudden Cardiac Death Misdiagnosed as Sudden Infant Death Syndrome (SIDS).

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a heart muscle disease characterised by arrhythmias of right ventricular origin, due to transmural fatty or fibrofatty replacement of atrophic myocardium. In general, the pathology is seldom diagnosed below the age of 10 years old. We report a case of a 5-year-old female child with Dandy-Walker Syndrome, who died suddenly the night after surgical replacement of the ventriculo-peritoneal shunting catheter. She never had any heart problems and the ECGs have never shown significant alterations. Histological examination of the common myocardium showed focal fatty replacement of the right ventricle, mostly evident in the subepicardial and mid-third of the free wall and of the interventricular septum. Finally, the histological examination of the atrio-ventricular node showed some anomalies of the conduction system. Probably the association of both abnormalities have caused a fatal arrhythmia, occurred after the surgical replacement of the ventriculo-peritoneal shunting catheter. [ABSTRACT FROM AUTHOR]